Peds - Limb Deficiencies Flashcards
Congenital limb deficiencies occur primarly during ______ trimester. What day?
1st trimester
Day 26 when mesoderm formation of the limb occurs and continues to differentiate until 8 weeks gestation.
Name the two risk factors for congenital limb deficiency
- thalidomide drug
- maternal diabetes
Name the 3 classification systems for congenital limb deficiency
which is the preferred?
- ISPO (international society for prosthetics and orthotics) classification system - PREFERRED
- Original (classic) classification
- Frantz classification
ISPO classification system classifies limb deficiencies as either ____ or _____.
What does each mean
Transverse: having no distal remaining portions. Named after segment beyond which there is no skeletal portion.
Longitudinal: having distal portion. name the bones that are affected. (Any bone not named is present and of normal form)
Original (classic) classification of limb deficiencies. What is the definition?
Acheiria
Acheiria - missing hand or foot
Original (classic) classification of limb deficiencies. What is the definition?
Adactyly
Adactyly - absent metacarpal or metatarsal
Original (classic) classification of limb deficiencies. What is the definition?
Amelia
abscence of a limb
Original (classic) classification of limb deficiencies. What is the definition?
aphalangia
absent finger or toe
Original (classic) classification of limb deficiencies. What is the definition?
hemimelia
absence of half of a limb
Original (classic) classification of limb deficiencies. What is the definition?
Phocomelia
flipper like appendage attached to the trunk.
Original (classic) classification of limb deficiencies. What is the definition?
meromelia
partial absence of alimb
Frantz classification describes limb deficiencies how?
terminal - representing the complete loss of the distal extremity
intercalary - denoting the absence of intermediate parts with preserved proximal and distal parts of the limb
these classifications are then divided into horizontal and longitudinal deficits
Given the classic (original) classification of limb deficiency, name the comparative Frantz and ISPO names
upper extremity amelia
Frantz: terminal transverse
ISPO: Transverse upper arm, total
Given the classic (original) classification of limb deficiency, name the comparative Frantz and ISPO names
Fibula hemimelia
Frantz: intercalary/normal foot
- Longitudinal/absent rays
- fibular deficiency
ISPO
Longitudinal fibular deficiency (total or partial)
Given the classic (original) classification of limb deficiency, name the comparative Frantz and ISPO names
upper extremity phocomelia
Frantz: complete upper extremity phocomelia, distal/absent radius ulna, proximal/absent humerus
ISPO: longitudinal total, humerus, ulna, radius. Carpal, or metacarpal, phalangeal (total or partial)
What is the incidence of congenital upper extremity deficiency?
4.1 per 10,000 lives births
which 3 upper extremity limb deficiencies have hereditary implications?
1 hand and feet deformity
2. central ray deficiency
3. adactyly involving the first four digits with the 5th intact.
_____ anomalies are associated with limb deficiences
craniofacial
What are the 5 syndromes associated with limb deficiencies?
- TAR syndrome
- Fanconi’s Syndrome
- Holt-Oram Syndrome
- Baller-Gerold Syndrome
- VACTERL (or VATER) Syndrome
What is TAR syndrome?
Thrombocytopenia with Absence of Radius
What is Fanconi syndrome?
Anemia and leukopenia developing at 5-6 yoa. plus limb deficiency
What is Holt-Oram Syndrome?
Congenital heart disease, especially atrial septal defects and tetralogy of fallot plus limb deficiency
What is Baller-Gerold Syndrome?
Craniosynostosis plus limb deficiency
What is VACTERL (or VATER) syndrome?
Vertebral defects
Anal atresia
Cardiac defects
Tracheo Esophageal fistula
Renal dysplasia
Limb deficiency
The most common congenital limb deficiency is _____
left terminal transradial deficiency
Fitting for the first prosthesis for unilateral limb deficiency should occur when:
when the child can sit unassisted (usually 6-7 months)
for unilateral limb deficiency, a more terminal device is provided around _____
11-13 months when the child begins to walk, performs some grasp and release activities, and has attention span of > 5 mins.
What is the typical initial transradial prosthesis comprised of
self-suspending design with supracondylar socket and a hand which is preferred by parents.
The _____ procedure reconstructs the forearm and creates a sensate prehensile surface for children with absent hands by separating the ulna and the radius in the forearm.
Krukenberg procedure
- rarely used with unilateral conditions due to dramatic cosmetic appearance
- indications include absent hands and visual impairment.
For transhumeral deficiency, fitting occurs when?
slightly later than transradial in order to achieve optimal results. A transhumeral prosthesis can be more of an encumberance to the infant
terminal devices are often issued when?
when a child begins to walk.
amputation revision is required in ____% of upper extremity limb deficiencies
10%
amputation revision
examples:
- radial club hand
- ulnar club hand
treatment is directed at centralization of the hand and reconstructing the thumb.
What is the Vikke procedure?
attaches a toe to the residual limb.
The higher the ______ the less likely the child will accept a prosthesis
higher the limb absence
transradial patients wear their prostheses more than transhumeral patients
_____ is the most common lower limb congenital deficiency. Bilateral occurs ____% of the time
fibular longitudinal deficiency (fibula hemimelia)
25% - unilateral creates a problem with limb length discrepency
If leg length inequality is severe with regard to congenital limb deficiency, a ____ procedure may be performed
symes amputation - fitting of a symes prosthesis.
_____ is the most common complication after amputation in the immature child.
This occurs most frequently on the ______
Terminal overgrowth at the transected end of the long bone
occurs most frequently on the
1. humerus
2. fibula
3. tibia
4 femur
(in order)
What are three complications associated with pediatric acquired amputations?
- bone spur formation
- development of adventitious bursae
- stump scarring requiring socket modifications
Name 3 advantages and 2 disadvantages of intra-operative prosthetic fitting of pediatric LE acquired amputations:
Advantages:
1. allows amputee to begin walking soon after surgery
2. Decreased edema and calf pain
3. good candidates include teenagers or young adults undergoing amputation for a tumor
Disadvantages
1. Weight-bearing restrictions and activity restrictions may put the stump at risk
2. poor candidates for this procedure include young children who do not understand restrictions, immunocompromised children, and kids with insensate limbs or infections.
Amputees preserve their energy expenditure by ____
decreasing their walking speed.
The older the child is at time of amputation, the greater the chance of _____
phantom pain, especially after age 10
The most common cause of pediatric acuired amputation is _____
trauma, occurring two times more frequently than disease-related amputation.
Most common type of acquired pediatric amputation?
90% single limb loss with 60% involving the lower extremity
Boys affected 3:2
The most frequent disease related cause of pediatric amputation is ____
tumors. highest incidence of malignancy occuring in the 12-21 age group.
____ and ____ are the most common pediatric cancers that could lead to amputation
- Osteogenic Sarcoma
- Ewings sarcoma
Aside from tumors, what are the 3 other disease related acquired amputation sources in kids?
- Vascular insuffiency - gangrene
- Neurologic disorders - neurofibromatosis with associated non-union of fracture
- emboli from meningococcemia may cause auto-amputation of limbs or digits and can affect all four limbs.
Varus foot, short leg, unstable knee, ankle, or both. Pathology?
longitudinal transtibial deficiency.
treatment of choice for longitudinal transtibial deficiency?
knee disarticulation.
30% of partial tibial deficiency occurs in ______ inheritance pattern
autosomal dominant
What is important in partial tibial deficiencies?
if the tibial segment is long enough, the surgeon creates a synostosis with the intact fibula and amputation of the foot. This provides a stable walking surface for the child without a prosthesis.
longitudinal deficiency of the femur is also known as _______ and occurs in 1 per _______ births
____% are bilateral
partial proximal femoral focal deficiency (PFFD)
1 per 50,000
10-15% are bilateral
What is the typical position of the femur in partial proximal femoral focal deficiency?
it is a longitudinal deficiency of the femur
typically is short, held in flexion, abduction, and external rotation.
70-80% of proximal femoral focal deficiency (PFFD) also have _______ deficiency
fibular
severe forms of proximal femoral focal deficiency (PFFD) require ______
fusion of the shortene femur to the tibia
removal of thef ot (symes amputation)
residual limb will accept appropriate above the knee prosthesis
what is a van ness rotation?
treatment of proximal femoral focal deficiency (PFFD) in which simulation of below-knee function is achieved by rotating the foot by 180 degreesso ankle motion can control the prosthesis .
- The lower limb prosthesis is fit in child with deficient limb when?
- normal heel-to-toe gait does not develop until:
- prosthetic heel-strike to toe off gait is not attained until ______ or when the child can demonstrate sustained one-legged standing
- a knee joint is usually added as early as ______
- when he or she is ready to pull up to standing position at 9-10 months.
- 2 years
- 5 years
- 18 months
1/3 of limb deficient children using a patellar tendon bearing prosthesis develop _____
patellar dislocation
Prostheses need to be replaced every _____ on the growing child
15-18 months
Performing joint disarticulations preserves
the epiphyseal growth plate
